Severe chronic neutropenia (SCN) is a very rare disease with around 650 people worldwide known to have the condition. SCN is a serous condition with a considerable morbidity and mortality if not treated. We hereby report a case of SCN which had repeated admissions to the hospital with severe neutropenia and high grade fever. We also review the literature elucidating some of the mechanisms and consequences of SCN ( e.g: the neutrophil elastase gene mutations and the risk of progression to myelodysplasia and acute leukemia) and the role of granulocyte-colony stimulating factor.